Follow-up assessment of pulmonary functions in mechanically ventilated children after discharge from pediatric intensive care unit: A developing country perspective

Background: There is a paucity of data on the pulmonary function tests (PFTs) in pediatric mechanically ventilated patients, especially in a developing country setting. Materials and Methods: This prospective study was carried out in the pediatric intensive care unit over 15 months. The PFTs were measured using spirometry at discharge, at 3 and 6 months. Results: Of 32 eligible children, 20 (mean age 9 ± 2.62 years) completed the 6-month follow-up. The most common indications for mechanical ventilation were respiratory (45%) and neurological (35%) causes. At the end of 6 months, 65% children had abnormal lung function (restrictive pattern). Patients with longer duration of ventilation, high peak pressures, and high fractional inhaled oxygen had a trend toward more abnormality in the lung function. Conclusions: Pediatric mechanically ventilated patients developed restrictive pulmonary defects after discharge that gradually improved over time. The majority was asymptomatic and reported no lifestyle limitations. There is a need for longer follow-up studies to assess the lung function and clinical condition postdischarge

Visual evoked potentials in children with type 1 diabetes mellitus

Background: Insulin-dependent (type 1) diabetes mellitus is associated with different degrees of neuropathies affecting peripheral as well as central neural pathways. The subclinical neurological involvement in diabetic children can be assessed by changes appearing in the electrophysiological parameters like Visual Evoked Potentials (VEPs). The objective of the study was to assess the effect of type I diabetes melitus on the visual evoked potentials in children.Methods: This cross-sectional case-control study was done on 30 children with type 1 diabetes mellitus of minimum two years duration as cases and 30 age and gender matched euglycemic children with normal HbA1c taken as controls. Visual Evoked Potentials (VEPs) were done on both the groups of children and the latencies (ms) of waves -N75, P100, N145 and amplitude (μV) of wave P100 were recorded. The data was compiled in the pre-designed proforma and statistically analysed using student t-test.Results: The increase in the mean latencies of waveforms of VEPs N75, P100 and amplitude P100 were found to be highly significant statistically (p<0.001) in both the eyes among the children with type 1 diabetes mellitus. The mean latency of waveform N145 was found to be statistically insignificant in the two groups (p>0.05).Conclusions: The type 1 diabetes does affect the visual pathways in children. Visual Evoked Potentials are helpful in the detection of early changes in the conduction across the neural pathways in the sub-clinical diseases

Empirical models for estimating monthly global solar radiation: a most comprehensive review and comparative case study in China

Global solar radiation is a core component of scientific research and engineering application across a broad spectrum. However, its measurement is limited by a small number of stations due to the technical and financial restricts. Estimating solar radiation with the meteorological variables using empirical models is of benefit to obtain solar radiation data at global scale. Yet, there are various options of available empirical models to select the most suitable one. This study conducted a most comprehensive collection and review of empirical models employing the commonly measured meteorological variables and geographic factors. A total of 294 different types of empirical models were collected and classified into 37 groups according to input attributes. Such collection built an empirical model library providing an overall overview of the developed empirical models in literatures. Furthermore, the collected models were calibrated and evaluated at three meteorological stations in the Three Gorges Reservoir Area in China. This study suggests that these model-comparing processes can assist the governments, scientists and engineers in tailoring the most fitted model for specific applications and in particular areas

Septate uterus with cervical and vaginal duplication: A rare Mullerian malformation

A rare case of Mullerian anomaly in a 26-year-old woman who presented with recurrent pregnancy loss and was found to have a longitudinal vaginal septum with cervical duplication and two endometrial cavities separated by a complete septum. The diagnosis of this unusual anomaly was aided by magnetic resonance imaging and was confirmed by hysterolaparoscopy. It was concluded that hysteroscopy aided in the diagnosis and treatment of this Mullerian defect with the highest quality of precision. Hysteroscopy therefore is considered gold standard in the management of septate uterus, thereby helping to regain the anatomical normality and because of its minimally invasive nature, it improves the reproductive outcome

Two unusual cases of PLA2G6-associated neurodegeneration from India

Phospholipase A2-associated neurodegeneration (PLAN) comprises of three disorders with overlapping presentations. The most common of these is classical or infantile-onset phospholipase A2-associated neurodegeneration, also known as infantile neuroaxonal dystrophy (INAD). Only 1 case of INAD has been reported from India till now. We report two genetically confirmed patients seen at a tertiary care pediatric hospital. Both these patients presented with infantile onset of neuroregression. We believe that INAD is underrecognized and underreported from India

Diabetes Mellitus as the Presenting Feature of Friedreich's Ataxia

Patients with Friedreich's ataxia (FA) are at an increased risk of developing diabetes mellitus and glucose intolerance. Diabetes usually develops many years after the initial presentation. We report an 8-year-old girl who initially presented with diabetic ketoacidosis and was treated as a case of insulin-dependent diabetes mellitus. Around a year later, she developed gait problems and ataxia. Cardiac involvement was detected on echocardiography. Genetic testing confirmed the diagnosis of FA. FA should be a diagnostic consideration in children presenting with diabetes and neurological issues, even with early presentation of the former. Early occurrence of diabetes and rapid progression of ataxia in this patient needs a better understanding of underlying genetic mechanisms

Giant axonal neuropathy: Clinical, radiological, and genetic features

Introduction: Giant axonal neuropathy (GAN) is an inherited neurodegenerative disorder caused by mutations in the GAN gene. It affects both the central and peripheral nervous systems. We discuss clinical, electrophysiological, radiological and genetic features in three new unrelated patients with GAN. Methods: Three pediatric patients with suspected GAN were included. The diagnosis was considered in patients with early onset polyneuropathy and characteristic hair with central nervous system involvement or suggestive neuroimaging findings. Biochemical, metabolic and electrophysiological investigations were performed. Diagnosis was confirmed by targeted sequencing of the GAN gene. Results: All the three patients were found to have biallelic mutations in GAN gene. Peripheral neuropathy, characteristic hair, and cerebellar dysfunction were present in all three while bony deformities, cranial nerve involvement and intellectual disability were seen variably. Neuroimaging showed a spectrum of findings which are discussed. Conclusion: GAN is a clinically and radiologically heterogeneous disease where genetic testing is necessary for a definite diagnosis and counselling. With facilities for testing becoming increasingly available, the spectrum is likely to expand further